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  • Investigating the purpose of prelamin A processing
  • Davies BS; Coffinier C; Yang SH; Barnes RH 2nd; Jung HJ; Young SG; Fong LG
  • Nucleus 2011[Jan]; 2 (1): 4-9
  • Lmna yields two major protein products in somatic cells, lamin C and prelamin A. Mature lamin A is produced from prelamin A by four posttranslational processing steps-farnesylation of a carboxyl-terminal cysteine, release of the last three amino acids of the protein, methylation of the farnesylcysteine, and the endoproteolytic release of the carboxyl-terminal 15 amino acids of the protein (including the farnesylcysteine methyl ester). Although the posttranslational processing of prelamin A has been conserved in vertebrate evolution, its physiologic significance remains unclear. Here we review recent studies in which we investigated prelamin A processing with Lmna knock-in mice that produce exclusively prelamin A (Lmna(PLAO)), mature lamin A (Lmna(LAO)) or nonfarnesylated prelamin A (Lmna(nPLAO)). We found that the synthesis of lamin C is dispensable in laboratory mice, that the direct production of mature lamin A (completely bypassing all prelamin A processing) causes no discernable pathology in mice, and that exclusive production of nonfarnesylated prelamin A leads to cardiomyopathy.
  • |Animals[MESH]
  • |Lamin Type A[MESH]
  • |Lamins/deficiency/genetics/*metabolism[MESH]
  • |Mice[MESH]
  • |Nuclear Proteins/*metabolism[MESH]
  • |Progeria/*metabolism/pathology[MESH]
  • |Protein Precursors/*metabolism[MESH]
  • |Protein Prenylation[MESH]
  • |Protein Processing, Post-Translational[MESH]





  • *{{pmid21647293}}
    *<b>[http://www.kidney.de/mlpefetch.php?search=21647293 Investigating the purpose of prelamin A processing ]</b> Nucleus 2011; 2(1) ; 4-9 Davies BS; Coffinier C; Yang SH; Barnes RH 2nd; Jung HJ; Young SG; Fong LG

        *21647293*

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    Nucleus

    4 1.2 2011